What are the other Names for this Condition? (Also known as/Synonyms)
- PPP (Purpura Pigmentosa Progressiva)
- Progressive Pigmentary Dermatosis of Schamberg
- Schamberg's Purpura
What is Schamberg Disease? (Definition/Background Information)
- Schamberg Disease is a skin condition that manifests as multiple papules and macules that are usually observed in the legs, which then slowly spreads to the rest of the body
- It is also known as Progressive Pigmentary Dermatosis of Schamberg and has no known cause. This chronic skin disorder is categorized in the group of pigmented purpuric dermatoses
- The skin discoloration occurs in patches, usually on the lower legs. The condition is not painful or frequently itchy. A skin biopsy may help confirm the diagnosis of Schamberg Disease
- It is difficult to treat Schamberg Disease, which is a progressive skin condition. They may also recur after suitable treatment and over time the condition becomes chronic. The mainline of treatment is the use of steroidal creams and lotions
- The prognosis of Schamberg Disease is generally good, though it may be present for several years. It may cause cosmetic issues and the affected individuals may face a lot of emotional stress
Who gets Schamberg Disease? (Age and Sex Distribution)
- Schamberg Disease can affect individuals of all ages
- Both males and females are affected; however, the condition is seen at a higher incidence in males
- There is no racial or ethnic predilection for this condition; it is observed worldwide
What are the Risk Factors for Schamberg Disease? (Predisposing Factors)
- Presently, there are no risk factors associated with Schamberg Disease
- It has been reported that viral infections may be a risk factor for the condition
It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.
Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.
What are the Causes of Schamberg Disease? (Etiology)
- The exact cause of Schamberg Disease is not well-established or understood. But a dysfunctional immune system may be a causative factor
- The skin discoloration occurs due to the inflammation of capillaries with leakage of red blood cells into the surrounding tissue. As the RBCs breakdown, the iron in the hemoglobin get deposited in the skin causing its discoloration
- The condition is not infectious and does not spread from one individual to another
What are the Signs and Symptoms of Schamberg Disease?
The clinical signs and symptoms of Schamberg Disease include the following:
- Localized (confined to a certain area), well-circumscribed areas of patches on the skin in the form of papules and macules, which are many in number
- The skin patches are occasionally itchy, but are usually not painful
- The onset of the patches takes place slowly, beginning from the lower legs. It then spreads to the rest of the body
How is Schamberg Disease Diagnosed?
The diagnosis of Schamberg Disease may involve the following procedures:
- The diagnosis is usually made by a good physical examination and evaluation of medical history
- Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
- Complete blood count (CBC) and other blood tests to rule out any infection
- Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. A skin biopsy is performed to rule out other similar conditions
Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.
What are the possible Complications of Schamberg Disease?
The complications of Schamberg Disease may include the following:
- Development of worsening and recurrent chronic skin lesions, which may be resistant to treatment
- The longstanding skin patches can cause emotional stress and psychological trauma in some individuals, leading to depression
- Rare cases of T-cell lymphoma have been reported to have developed due to Schamberg Disease
How is Schamberg Disease Treated?
The treatment of Schamberg Disease is difficult and the condition is slowly progressive and chronic in nature. The lesions may recur after a period of time, sometimes after the condition gets better. The treatment provided may only help in improving the symptoms, but there is no cure for Schamberg Disease.
- The application of topical steroid creams and lotions is usually beneficial
- The presence of chronic skin lesions may cause psychological discomfort; individuals may require reassurance to help with stress and anxiety
- Antibiotic therapy is not helpful in the treatment of this skin condition
Despite therapy, the skin lesions persist for a long period of time, resulting in a chronic skin disorder.
How can Schamberg Disease be Prevented?
There is currently no effective means of prevention of Schamberg Disease, because the cause of this condition is unknown.
What is the Prognosis of Schamberg Disease? (Outcomes/Resolutions)
- The skin lesions in Schamberg Disease are not life-threatening, however they can cause cosmetic concerns in many individuals
- With appropriate treatment the condition may get better
Additional and Relevant Useful Information for Schamberg Disease:
There are 3 types of pigmented purpuric dermatosis (PPD), which include:
- Schamberg Disease
- Gougerot-Blum Disease
- Majocchi Disease
What are some Useful Resources for Additional Information?
American Academy of Dermatology
930 E. Woodfield Road Schaumburg, IL 60173
Phone: (866) 503-SKIN (7546)
Fax: (847) 240-1859
References and Information Sources used for the Article:
Wolff, K., & Johnson, R. A. (2009). Fitzpatrick's color atlas and synopsis of clinical dermatology. McGraw-Hill Medical.
Thiers, B. H. (1989). Year Book of Dermatology 1988. Archives of Dermatology, 125(8), 1150.
Burns, T., & Breathnach, S. (1992). Rook's Textbook of dermatology Vol 4. London: Blackwell Scientific Publications, 1992.
Bolognia, J. L., Schaffer, J. V., Duncan, K. O., & Ko, C. J. (2014). Dermatology Essentials E-Book. Elsevier Health Sciences.
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First uploaded: Oct. 12, 2015
Last updated: April 13, 2016
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